Chapter

General Principles of Medical Management

R. E. Williams

in The Neuronal Ceroid Lipofuscinoses (Batten Disease)

Second edition

Published on behalf of Oxford University Press

Published in print March 2011 | ISBN: 9780199590018
Published online November 2012 | e-ISBN: 9780191753459 | DOI: http://dx.doi.org/10.1093/med/9780199590018.003.0005

Series: Contemporary Neurology Series

General Principles of Medical Management

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Despite the findings of extensive basic scientific and clinical research, the NCLs remain a group of neurodegenerative disorders for which there is currently no cure. They share many features with other paediatric progressive neurological disorders. They are, however, characterized by some unique and complex clinical problems. Medical management should follow general principles and good practice, but should also incorporate an appreciation of the special needs of this group. Parents and families are also often keen to explore complementary and experimental therapeutic approaches alongside traditional medicine.

Much can be done to alleviate symptoms using the approaches of traditional medicine. Clinical care aims to minimize the impact of symptoms such as seizures, maintain and promote skills, and to optimize quality of life for affected individuals and their families. Even when potentially curative therapies become available, there will still be an important place for holistic and symptomatic medical care. A multidisciplinary and multiagency (health, education, and social services) approach is essential and must be child-centred. The World Health Organization (http://www.who.int/cancer/palliative/definition/en/) has developed a definition of palliative care for children who are suffering from a life-limiting illness: Palliative care for children is the active total care of the child’s body, mind and spirit, and also involves giving support to the family. It begins when illness is diagnosed, and continues regardless of whether or not a child receives treatment directed at the disease. Health providers must evaluate and alleviate a child’s physical, psychological, and social distress. Effective palliative care requires a broad multidisciplinary approach that includes the family and makes use of available community resources; it can be successfully implemented even if resources are limited. It can be provided in tertiary care facilities, in community health centres and even in children’s homes.

In this definition of palliative care, a holistic approach of patient care is clearly described.

Much of current medical, nursing, and therapy practice in the NCLs does not have a solid evidence base. Much of what is presented here is based on personal practice and narrative or anecdotal evidence from professional colleagues both in the UK and internationally.

Chapter.  2696 words. 

Subjects: Neurology

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